Abstract
In sympathetic neurocirculatory failure (SNF), reflexive sympathetically mediated cardiovascular stimulation does not compensate for decreased cardiac filling. This explains orthostatic hypotension in chronic primary autonomic failure (CPAF). During phase 2 of the Valsalva maneuver (phase 2_L), blood pressure increases from its peak. During phase 4, blood pressure normally “overshoots” the baseline. Because these changes depend on sympathetically mediated cardiovascular stimulation, a progressive decrease in pressure during phase 2 and absence of the overshoot in phase 4 may indicate SNF. Moreover, because beat-to-beat blood pressure can be measured noninvasively using a photoplethysmographic or tonometric device, evaluating reflexive pressure responses might enable noninvasive diagnosis of SNF. This study assessed the relative frequencies of abnormal phase 2_L and phase 4 blood pressure in patients with CPAF and orthostatic hypotension and whether noninvasive measurement of beat-to-beat blood pressure can be used to diagnose SNF in patients. Twenty patients with chronic primary autonomic failure and orthostatic hypotension and 50 comparison patients, including several with CPAF but lacking orthostatic hypotension, underwent arterial pressure monitoring during performance of the Valsalva maneuver. Of the 20 patients with CPAF and orthostatic hypotension, all had an abnormal phase 2_L or phase 4 pressure response (sensitivity 100%), whereas only 3 of the 50 comparison patients had an abnormal response in either phase (specificity 94%). Seventeen patients with CPAF and orthostatic hypotension had abnormal responses in both phases (sensitivity 85%), but none of the comparison patients had such findings in both phases (specificity 100%). Of 13 patients in whom beat-to-beat blood pressure was recorded simultaneously invasively and noninvasively, all had abnormal blood pressure responses during phase 2_L and phase 4, whereas none of 29 comparison patients had such symptoms. Detection of abnormal blood pressure responses during phase 2_L or phase 4 of the Valsalva maneuver is a highly sensitive test for SNF. Abnormal pressure during these phases appears to identify SNF specifically. Noninvasive measurements can detect both of these abnormalities.
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References
Beers, MH, Berkow R.The Merck manual of diagnosis and therapy, Whitehouse Station, NJ: Merck Research Laboratories; 1999.
Ziegler MG, Lake CR, Kopin IJ. The sympathetic-nervous-system defect in primary orthostatic hypotension.N Engl J Med 1977; 296:293–297.
Mathias CJ. The classification and nomenclature of autonomic disorders-ending chaos, resolving conflict and hopefully achieving clarity.Clin Auton Res 1995; 5:307–310.
Gilman S, Low P, Quinn N, et al. Consensus statement on the diagnosis of multiple system atrophy.Clin Auton Res 1998; 8:359–362.
Goldstein DS, Holmes C, Cannon RO, et al. Sympathetic cardioneuropathy in dysautonomias.N Engl J Med 1997;336:696–702.
Delius W, Hagbarth KE, Hongell A, et al. Manoeuvres affecting sympathetic outflow in human muscle nerves.Acta Physiol Scand 1972;84:82–94.
Johnson RH, Lambie DG, Spalding JMK.Neurocardiology: the interrelationships between dysfunction in the nervous and cardiovascular systems. Philadelphia: WB Saunders; 1984.
Sharpey-Schafer EP. Effect of respiratory acts on the circulation.Handbook of Physiology Section 2: Circulation 1965;3:pp. 1875–1886.
Jordan J, Shannon JR, Black BK, et al. N(N)-nicotinic blockade as an acute human model of autonomic failure.Hypertension 1998; 31:1178–1184.
Sandroni P, Benarroch EE, Low PA. Pharmacological dissection of components of the Valsalva maneuver in adrenergic failure.J Appl Physiol 1991;71:1563–1567.
Low PA. Autonomic nervous system function.J Clin Neurophysiol 1993;10:14–27.
Benarroch EE, Sandroni P, Low PA. The Valsalva maneuver. In:Clinical autonomic disorders. Low PA, ed. Boston: Little, Brown and Company; 1993. pp. 209–215.
Parati G, Casadei R, Groppelli A, et al. Comparison of finger and intra-arterial blood pressure monitoring at rest and during laboratory testing.Hypertension 1989;13:647–655.
Imholz BP, van Montfrans GA, Settels JJ, et al. Continuous noninvasive blood pressure monitoring: reliability of Finapres device during the Valsalva manoeuvre.Cardiovasc Res 1988; 22:390–397.0
Bos WJ, Imholz BP, van Goudoever J, et al. The reliability of noninvasive continuous finger blood pressure measurement in patients with both hypertension and vascular disease.Am J Hypertens 1992;5:529–535.
Imholz BP, Wieling W, Langewouters GJ, van Montfrans GA. Continuous finger arterial pressure: utility in the cardiovascular laboratory.Clin Auton Res 1991;1:43–53.
Rongen GA, Bos WJ, Lenders JW, et al. Comparison of intrabrachial and finger blood pressure in healthy elderly volunteers.Am J Hypertens 1995;8:237–248.
Braune S, Auer A, Schulte-Monting J, et al. Cardiovascular parameters: sensitivity to detect autonomic dysfunction and influence of age and sex in normal subjects.Clin Auton Res 1996; 6:3–15.
Niimi Y, Ieda T, Hirayama M, et al. Clinical and physiological characteristics of autonomic failure with Parkinson's disease.Clin Auton Res 1999;9:139–144.
Goldstein DS. A new sign of sympathetic neurocirculatory failure: premature ventricular contraction as a “one-beat Valsalva maneuver”.Clin Auton Res 2000;10:63–67.
Cohen J, Low P, Fealey R, et al. Somatic and autonomic function in progressive autonomic failure and multiple system atrophy.Ann Neurol 1987;22:692–699.
Van Egmond J, Hasenbos M, Crul JF. Invasive v. non-invasive measurement of arterial pressure.Br J Anaesth 1985; 57:434–444.
Warren JH, Jaffe RS, Wraa CE, et al. Effect of autonomic blockade on power spectrum of heart rate variability during exercise.Am J Physiol 1997;273:R495-R502.
Prinz-Zaiss M, Yeap AN, Moguilevski V, et al. Power spectral analysis of heart rate variability during graded head-up tilting in patients with vasodepressor syncope.Clin Exp Pharmacol Physiol 1995;22:472–474.
Taylor AA, Davies AO, Mares A, et al. Spectrum of dysautonomia in mitral valvular prolapse.Am J Med 1989;86:267–274.
Boulos M, Barron S, Nicolski E, et al. Power spectral analysis of heart rate variability during upright tilt test: a comparison of patients with syncope and normal subjects.Cardiology 1996; 87:28–32.
Baharav A, Mimouni M, Lehrman-Sagie T, et al. Spectral analysis of heart rate in vasovagal syncope: the autonomic nervous system in vasovagal syncope.Clin Auton Res 1993;3:261–269.
Murata K, Sumida Y, Murashima S, et al. A novel method for the assessment of autonomic neuropathy in type 2 diabetic patients: a comparative evaluation of 123I-MIBG myocardial scintigraphy and power spectral analysis of heart rate variability.Diabet Med 1996;13:266–272.
Omboni S, Parati G, Di Rienzo M, et al. Blood pressure and heart rate variability in autonomic disorders: a critical review.Clin Auton Res 1996;6:171–182.
Lombardi F, Guzzetti S, Rimoldi O, et al. Power spectral analysis of heart rate and arterial pressure variabilities as a marker of sympatho-vagal interaction in man and conscious dog.Circ Res 1986;59:178–193.
Tanabe T, Iwamoto T, Fusegawa Y, et al. Alterations of sympathovagal balance in patients with hypertrophic and dilated cardiomyopathies assessed by spectral analysis of RR interval variability.Eur Heart J 1995;16:799–807.
Hojgaard MV, Holstein-Rathlou NH, Agner E, et al. Dynamics of spectral components of heart rate variability during changes in autonomic balance.Am J Physiol 1998;275:H213-H219.
Kaye DM, Esler M, Kingwell B, et al. Functional and neurochemical evidence for partial cardiac sympathetic reinnervation after cardiac transplantation in humans.Circulation 1993; 88:1110–1118.
Randall DC, Brown DR, Raisch RM, et al. SA nodal parasympathectomy delineates autonomic control of heart rate power spectrum.Am J Physiol 1991;260:H985-H988.
Low PA, Caskey PE, Tuck RR, et al. Quantitative sudomotor axon reflex test in normal and neuropathic subjects.Ann Neurol 1983; 14:573–580.
Magnifico F, Misra VP, Murray NM, et al. The sympathetic skin response in peripheral autonomic failure-evaluation in pure failure, pure cholinergic dysautonomia and dopamine-betahydroxylase deficiency.Clin Auton Res 1998;8:133–138.
Goodall M. Innervation and inhibition of eccrine and apocrine sweating in man.J Clin Pharmacol 1970;10:235–246.
Low PA, Denq JC, Opfer-Gehrking TL, et al. Effect of age and gender on sudomotor and cardiovagal function and blood pressure response to tilt in normal subjects.Muscle Nerve 1997; 20:1561–1568.
Biaggioni I, Goldstein DS, Atkinson, et al. Dopamine-betahydroxylase deficiency in humans.Neurology 1990;40:370–373.
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Goldstein, D.S., Tack, C. Noninvasive detection of sympathetic neurocirculatory failure. Clinical Autonomic Research 10, 285–291 (2000). https://doi.org/10.1007/BF02281111
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DOI: https://doi.org/10.1007/BF02281111